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    Reports

    Atopic Keratoconjunctivitis inChildren: Clinical Features andDiagnosis

    Distinguishing between vernal keratoconjunctivitis (VKC) andatopic keratoconjunctivitis (AKC) can be challenging. Historically,AKC is rarely recognized as a diagnostic entity before puberty andis thought to occur predominantly in adults. If a young patient wereto present with AKC-like symptoms and atopic dermatitis, theymight be diagnosed with VKC.1 The aim of this report was toestablish guidelines for distinguishing diagnosis between AKCand VKC.

    We conducted a case survey of 23 pediatric patients with severekeratoconjunctivitis and atopic dermatitis who presented at 4 cen-ters between 2011 and 2013. Mean ages at onset of symptoms andat initial presentation to an ophthalmologist were 5.2 and 8.1 years,respectively. All patients suffered from eczema and conjunctivitis/

    keratitis, and the majority (74%) had a family history of atopy andwere affected by asthma and allergic rhinitis. The clinical featuresof patients with AKC are presented in Table 1 and Figure 1(available at www.aaojournal.org). The most prevalent clinicalfeatures within this study were conjunctival hyperemia andeczema, both of which were reported in 96% of patients. Otherclinical features include follicles, keratitis, and thickened dryskin, which were present in 83% of patients. Papillae,DennieeMorgan folds of the lower lid and blepharitis werepresent in 65% of patients. Other clinical features such as giantpapillae (>1 mm diameter), DennieeMorgan double folds of theupper lid, pseudoptosis, inltration of the inferior conjunctiva,HornereTrantas dots, and madarosis were present in 39% ofpatients.

    In this report, AKC in children is dened as the presence ofsevere allergic conjunctivitis with atopic dermatitis that is diag-nosed before 16 years of age. This may be accompanied by thepresence or absence of the following clinical features: conjunctivalhyperemia with eczema, madarosis, and blepharitis, with theabsence of HornereTrantas dots and giant papillae. The clinicalfeatures described here may be used to dene a grading system forthe identication of AKC in children. No single clinical featureviewed in isolation can accurately differentiate between AKC andVKC.

    Vernal keratoconjunctivitis is a rare, yet severe, form of allergicconjunctivitis, estimated to affect 3.2 out of every 10 000 in-habitants in western Europe.2 Vernal keratoconjunctivitis generally

    ends at puberty; however, in some cases, it is thought that VKCmay evolve into AKC in adulthood.2 In the absence of typicalclinical signs of VKC, a child with atopic dermatitis may bediagnosed with AKC and not VKC.1 Vernal keratoconjunctivitispresents with highly specic symptoms, such as photophobia,tearing, pseudoptosis, thick mucus discharge, and shield ulcers.2

    Children with VKC may present withatopic dermatitis; however,it is not a prerequisite for diagnosis.1 In contrast, evidence ofatopic dermatitis must be present for a diagnosis of AKC to bemade.1

    In accordance with the ndings reported herein, AKC in chil-dren may be more prevalent than initially believed; in an epide-miologic study of 134 patients with allergic conjunctivitis, 55% ofpatientswith AKC reported an onset of symptoms before 10 yearsof age.3 In Japanese populations, VKC cases with any history of

    atopic dermatitis is diagnosed as AKC, regardless of patientage.4 In Europe, these cases would only be diagnosed as AKC ifthe symptoms continuedpast puberty and occurred concurrentlywith keratoconjunctivitis.1 The multifactorial assessment of keyclinical signs presented, taking into consideration the presence ofAKC-related clinical features and the absence of VKC-relatedclinical features, in combination with a history of eczema andconjunctivitis/keratitis, may promote accurate diagnosis of AKC inchildren. Atopic keratoconjunctivitis and VKC differ in relation tospecic treatment needs. The dermatologic manifestations of AKCneed to be treated with emollients and demulcents and, if neces-sary, corticosteroids. Furthermore, although immunomodulatorssuch as cyclosporine and tacrolimus are effective treatments for

    both severe AKC and VKC, lower concentrations of these drugsmay be required in patients with AKC.5 These differences in theoptimal treatment of AKC and VKC highlight the importance ofearly and accurate diagnosis, in informing effective treatmentstrategies and improving patient outcomes.

    DOMINIQUEBRMOND-GIGNAC,MD, PHD1,2

    KENK. NISCHAL, MD, PHD3,4

    BRUNOMORTEMOUSQUE, MD, PHD5

    EVAGAJDOSOVA, MD, PHD3

    DAVIDB. GRANET, MD, PHD6

    FRDRICCHIAMBARETTA, MD, PHD71

    Pediatric Ophthalmology Department, University Hospital Necker-Enfants Malades, Paris, France; 2CNRS Unit FR3636, Paris V

    University, Paris, France; 3

    Clinical and Academic Department of

    Ophthalmology, Great Ormond Street Hospital for Children, London,

    UK; 4Pediatric Ophthalmology, Strabismus and Adult Motility UPMC

    Eye Center, Childrens Hospital of Pittsburgh, Pittsburgh,

    Pennsylvania; 5Ophthalmology Department, University Hospital of

    Pontchaillou, Rennes, France; 6Ratner Childrens Eye Center and

    Shiley Eye Center, University of California, San Diego, California;7

    Ophthalmology Department, University Hospital Gabriel Montpied,

    Clermont-Ferrand, France

    Financial Disclosure(s): The authors have made the following disclo-sures: Allergan, Inc. (Irvine, CA) funded medical writing support buthad no role in the design or conduct of this research.

    Author Contributions:Conception and design: Brmond-Gignac, Nischal, Mortemousque,Gajdosova, Granet, ChiambarettaAnalysis and interpretation: Brmond-Gignac, Nischal, Mortemousque,Gajdosova, Granet, ChiambarettaData collection: Brmond-Gignac, Nischal, Mortemousque, Gajdosova,Granet, ChiambarettaOverall responsibility: Brmond-Gignac, Nischal, Mortemousque,Gajdosova, Granet, Chiambaretta

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    Correspondence:Dominique Brmond-Gignac, Pediatric Ophthalmology Department,AP-HP, University Hospital Necker-Enfants Malades, 149 Rue deSvres, 75015 Paris, France. E-mail: [email protected]

    References

    1. Calonge M, Herreras JM. Clinical grading of atopic kera-

    tonconjunctivitis. CurrOpinAllergy ClinImmunol2007;7:4425.2. Bremond-Gignac D, Donadieu J, Leonardi A, et al. Prevalence

    of vernal keratoconjunctivitis: a rare disease? Br J Ophthalmol2008;92:1097102.

    3. Belfort R, Marbeck P, Hsu C, et al. Epidemiological study of134 subjects with allergic conjunctivitis. Acta OphthalmolScand 2000;78:3840.

    4. Ebihara N, Ohashi Y, Uchio E, et al. A large prospectiveobservational study of novel cyclosporine 0.1%aqueous ophthalmic solution in the treatment of severeallergic conjunctivitis. J Ocul Pharmacol Ther 2009;25:36572.

    5. Erdinest N, Solomon A. Topical immunomodulators in themanagement of allergic eye diseases. Curr Opin Allergy ClinImmunol 2014;14:45763.

    Table 1. Clinical Features of Patients (n 23) with AtopicKeratoconjunctivitis

    Clinical Features Present, n (%)

    Conjunctival hyperemia 22 (96)Follicles 19 (83)Papillae upper/lower 19 (83)Giant papillae (> 1 mm diameter) 14 (61)HornereTrantas dots (limbus) 9 (39)Swelling of the limbus 9 (39)Chemosis (conjunctival) 6 (26)Limbaldneovascularization/corneal opacication 11 (48)Keratitis (supercial punctate keratitis, shield ulcer,

    ulcerations and corneal erosions)20 (87)

    Inltration of inferior conjunctiva 12 (52)DennieeMorgan

    Double fold lower lid 18 (78)Double fold upper lid 13 (57)

    Pseudoptosis 12 (52)Facial cutaneous ssures (ears and canthus) 15 (65)Anterior blepharitis 15 (65)Posterior blepharitis 15 (65)Eczema, thickened and dry eyelid 22 (96)Thickened and dry skin

    Facial 20 (87)Body 19 (83)

    Madarosis 10 (44)

    Ophthalmology Volume -, Number -, Month 2015

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